Lauronen L, Heikkilä E, Autti T, Sainio K, Huttunen J, Aronen H J, Korvenoja A, Ilmoniemi R J, Santavuori P
Children's Hospital, University of Helsinki, Finland.
J Child Neurol. 1997 Sep;12(6):355-60. doi: 10.1177/088307389701200603.
The present study evaluated neurophysiologic function of the primary sensorimotor cortex in juvenile neuronal ceroid lipofuscinosis. A 122-channel magnetometer, which allowed studies of the somatosensory system in millimeter and millisecond precision, was used to record somatosensory evoked magnetic fields to median nerve stimulation from 10 patients and their matched control subjects. In both patients and controls, the somatosensory evoked magnetic fields from primary sensorimotor area typically consisted of N20m, P35m, and P60m deflections. In the patients, N20m was significantly delayed, whereas P35m peaked earlier than in the control subjects. The source strengths for N20m and P35m were greater in the patients than in the controls. Both deflections showed a significant positive correlation with the disease duration: the sources were stronger in the older patients than in the younger ones. P60m deflections were normal or reduced in the patients. The results indicated increased thalamocortical excitability in the sensorimotor cortex in juvenile neuronal ceroid lipofuscinosis.
本研究评估了青少年神经元蜡样脂褐质沉积症患者初级感觉运动皮层的神经生理功能。使用一台122通道磁力计来记录10名患者及其匹配的对照受试者在正中神经刺激下的体感诱发电场，该磁力计能够以毫米和毫秒精度对体感系统进行研究。在患者和对照受试者中，初级感觉运动区的体感诱发电场通常由N20m、P35m和P60m偏转组成。在患者中，N20m明显延迟，而P35m的峰值比对照受试者更早出现。患者中N20m和P35m的源强度比对照受试者更大。这两种偏转均与疾病持续时间呈显著正相关：年龄较大的患者源强度比年龄较小的患者更强。患者的P60m偏转正常或降低。结果表明，青少年神经元蜡样脂褐质沉积症患者感觉运动皮层的丘脑皮质兴奋性增加。
